LYSINURIC PROTEIN INTOLERANCE WITH CHRONIC INTERSTITIAL LUNG-DISEASE AND PULMONARY CHOLESTEROL GRANULOMAS AT ONSET

被引：24

作者：

KEREM, E

ELPELG, ON

SHALEV, RS

ROSENMAN, E

BARZIV, Y

BRANSKI, D

机构：

[1] HADASSAH MED CTR, DEPT PATHOL, JERUSALEM, ISRAEL

[2] HADASSAH MED CTR, DEPT RADIOL, JERUSALEM, ISRAEL

[3] SHAARE ZEDEK MED CTR, METAB UNIT, IL-91030 JERUSALEM, ISRAEL

[4] SHAARE ZEDEK MED CTR, NEUROL UNIT, IL-91030 JERUSALEM, ISRAEL

来源：

JOURNAL OF PEDIATRICS | 1993年 / 123卷 / 02期

关键词：

D O I：

10.1016/S0022-3476(05)81703-2

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Hyperammonemia and encephalopathy developed in an 11-year-old girl with chronic interstitial lung disease and cholesterol casts in her lung biopsy specimen. She had decreased plasma levels of ornithine, lysine, and arginine and excessive urinary excretion of lysine and arginine, consistent with the diagnosis of lysinuric protein intolerance. Analysis of plasma and urinary amino acids should be considered in the diagnostic evaluation of patients with interstitial lung disease of uncertain origin.

引用

页码：275 / 278

页数：4

共 13 条

[1]

DESJEUX JF, 1980, J CLIN INVEST, V65, P1382, DOI 10.1172/JCI109802

[2] PULMONARY PARENCHYMAL CHOLESTEROL-ESTER GRANULOMAS IN PATIENTS WITH PULMONARY HYPERTENSION [J].